Background: Female reproductive tract abnormalities are generally encountered in 4%-7% of women. Various combinations have been described in the literature. Our case reports a rare female genital tract anomaly of the distal vagina combined with uterus didelphys. Case Presentation: A 15-year-old girl with lower vaginal atresia associated with uterus didelphys presented with cyclical progressively worsening abdominal pain and primary amenorrhea. Pelvic ultrasound (US) and magnetic resonance imaging (MRI) illustrated two separated uterine cavities associated with a distended fluid-filled structure ending in a blind pouch at the lower margin of the vagina. Conclusion: The presence of congenital anomalies of the female urogenital system may be suspected in the case of cyclical abdominal pain in young girls with primary amenorrhea. The use of US as a primary approach is recommended. MRI is preferred to specify the height and extent of vaginal aplasia as long as to guide the choice of the most appropriate surgical technique.

Keywords: vaginal atresia, hematocolpos, female genital malformation, uterus didelphys